title: Malignant arrhythmogenic right ventricular dysplasia/cardiomyopathy with a normal 12-lead electrocardiogram: a rare but underrecognized clinical entity.
abstract: BACKGROUND
  In Arrhythmogenic Right Ventricular Dysplasia/Cardiomyopathy (ARVD/C), a normal electrocardiogram (ECG) is considered reassuring. However, some patients with ARVD/C experiencing ventricular arrhythmias have a normal ECG.
  
  
  OBJECTIVE
  To estimate how often patients with ARVD/C experiencing ventricular arrhythmias have a normal ECG during sinus rhythm, and to provide a clinical profile of these patients.
  
  
  METHODS
  We included 145 patients with ARVD/C experiencing a documented sustained ventricular arrhythmia. Conventional 12-lead sinus rhythm ECGs within 6 months of the event were reviewed for diagnostic Task Force Criteria (TFC). ECGs were classified as abnormal (≥1 TFC), nonspecific (abnormal, no TFC), or normal. Cardiologic investigations within 6 months of the event were evaluated as per TFC in those with a nonspecific or normal ECG.
  
  
  RESULTS
  The ECG was nonspecific or normal in 17 of 145 (12%) subjects. Mean age of these patients was 41.3 ± 12.4 years and 14 (82%) were men, comparable to those with an abnormal ECG. Most patients with a nonspecific or normal ECG showed ≥1 TFC on Holter monitoring (n = 9 of 10) and signal-averaged ECG (n = 4 of 5), and all had nonsustained ventricular tachycardia recorded. Among 15 patients who underwent structural evaluation, 11 (73%) showed structural TFC (9 major and 2 minor).
  
  
  CONCLUSIONS
  Although most patients with ARVD/C experiencing arrhythmias have an abnormal ECG, a nonspecific or normal ECG does not preclude ARVD/C diagnosis. All patients with a nonspecific or normal ECG had alternative evidence of disease expression. These results alert the physician not to rely exclusively on ECG in ARVD/C, but to assess arrhythmic risk by comprehensive clinical evaluation.
authors.affiliation: Department of Cardiology, University Medical Center Utrecht, Utrecht, The Netherlands; Department of Medicine, Division of Cardiology, Johns Hopkins University School of Medicine, Baltimore, Maryland.
authors.forename: Anneline S J M
authors.initials: AS
authors.lastname: te Riele
authors.forename: Cynthia A
authors.initials: CA
authors.lastname: James
authors.forename: Aditya
authors.initials: A
authors.lastname: Bhonsale
authors.forename: Judith A
authors.initials: JA
authors.lastname: Groeneweg
authors.forename: Christian F
authors.initials: CF
authors.lastname: Camm
authors.forename: Brittney
authors.initials: B
authors.lastname: Murray
authors.forename: Crystal
authors.initials: C
authors.lastname: Tichnell
authors.forename: Jeroen F
authors.initials: JF
authors.lastname: van der Heijden
authors.forename: Dennis
authors.initials: D
authors.lastname: Dooijes
authors.forename: Daniel P
authors.initials: DP
authors.lastname: Judge
authors.forename: Richard N W
authors.initials: RN
authors.lastname: Hauer
authors.forename: Harikrishna
authors.initials: H
authors.lastname: Tandri
authors.forename: Hugh
authors.initials: H
authors.lastname: Calkins
mesh_terms: D000328:Adult; D019571:Arrhythmogenic Right Ventricular Dysplasia / Q000150:complications* / Q000503:physiopathology*; D015331:Cohort Studies; D042241:Early Diagnosis; D015716:Electrocardiography, Ambulatory; D005260:Female; D006801:Humans; D008297:Male; D008875:Middle Aged; D011237:Predictive Value of Tests; D018570:Risk Assessment; D012307:Risk Factors; D017180:Tachycardia, Ventricular / Q000175:diagnosis* / Q000209:etiology / Q000503:physiopathology*; D055815:Young Adult
keywords: ARVD/C; Arrhythmogenic Right Ventricular Dysplasia/Cardiomyopathy; CMR; Cardiomyopathy; Concealed; Diagnosis; ECG; Electrocardiogram; PKP2; PVC; Plakophilin-2; RV; Risk stratification; SAECG; TAD; TFC; Task Force Criteria; VF; VT; Ventricular arrhythmias; cardiac magnetic resonance; electrocardiogram/electrocardiographic; premature ventricular complex; right ventricular; signal-averaged electrocardiogram; terminal activation duration; ventricular fibrillation; ventricular tachycardia